EWS-FLI (ets) Fusion Transcripts

Definition

The EWS-FLI1 fusion transcript is the result of a balanced reciprocal chromosomal translocation between chromosomes 11 and 22, which fuses the EWS gene in chromosome 22 to the FLI1 gene in chromosome 11. This fusion transcript is detected in approximately 85 % of cases of the Ewing sarcoma family of tumors and is considered a tumor-specific molecular rearrangement, therefore useful for diagnosis, prognosis, and presumably specific therapeutics. Approximately 10 % of Ewing tumors have fusions involving EWS and ERG genes. An additional 5 % bear fusions between EWS and other less frequent genes.

Characteristics

Structure

Chromosomal translocations result in the genesis of chimeric genes, encoding hybrid transcripts and novel fusion proteins. Many fusion proteins contain juxtaposed functional domains usually found in separate proteins. The EWS-FLI1 fusion protein contains the aminoterminal domain of EWS and the carboxyterminal region of FLI1. EWSgene is an RNA-binding protein,...